Pathological diagnosis of extramammary Paget’s disease of the anogenital region

Extramammary Paget’s disease is a rare tumor, with the vulva as the most common site of occurrence. Although tumors in this region are mainly seen by gynecologists, they can also be encountered occasionally in dermatological practices. The tumor often draws attention with itching. Due to the non-specific morphology and symptoms, diagnosis may take up to several years. While the primary form originates in the skin, the secondary extramammary Paget’s disease is associated with an internal tumor. Pathological examination plays an indispensable role in recognizing the disease and clarifying its background, which helps the clinician in establishing further diagnostic steps and therapeutic plans.